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Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm
YAJEM-56683; No of Pages 6 American Journal of Emergency Medicine xxx (2017) xxx–xxx
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report Moritz Weigeldt, MD a,⁎, Sabine Lahmann, MD b, Konstantin Krieger, MD c, Sebastian Buttenberg, MD d, Volker Stephan, MD, PhD e, Brigitte Stiller, MD, PhD f, Dirk Stengel, MD, PhD MSc g a
Center for Clinical Research and Department of Anesthesiology, Critical Care and Pain Medicine, Unfallkrankenhaus Berlin, Warener Str. 7, 12683 Berlin, Germany Department of Anesthesiology, Critical Care and Pain Medicine, Unfallkrankenhaus Berlin, Warener Str. 7, 12683 Berlin, Germany Department of Internal Medicine and Cardiology, Unfallkrankenhaus Berlin, Warener Str. 7, 12683 Berlin, Germany d Department of Pediatrics, Sana Klinikum Lichtenberg, Fanningerstraße 32, 10365 Berlin, Germany e Department of Pediatrics, Sana Klinikum Lichtenberg, Fanningerstraße 32, 10365 Berlin, Germany f Department of Congenital Heart Defects and Pediatric Cardiology, Heart Center University of Freiburg, Mathildenstr. 1, 79106 Freiburg, Germany g Center for Clinical Research, Unfallkrankenhaus Berlin, Warener Str. 7, 12683 Berlin, Germany b c
a r t i c l e
i n f o
Article history: Received 8 March 2017 Received in revised form 9 May 2017 Accepted 10 May 2017 Available online xxxx Keywords: Pediatric Cardio-pulmonary resuscitation Out of hospital cardiac arrest - shockable rhythm Automated external defibrillator Coronary artery agenesis
a b s t r a c t Background: To illustrate a rare cause of out-of-hospital cardiac arrest in children, its differential diagnoses, emergency and subsequent treatment at various steps in the rescue chain, and potential outcomes. Case presentation: A 4-year-old boy with unknown agenesis of the left coronary ostium sustained out-of-hospital cardiac arrest. Bystander cardio-pulmonary resuscitation was initiated and defibrillation was performed via an automated external defibrillator (AED) shortly after paramedics arrived at the scene, restoring sinus rhythm and spontaneous circulation. After admission to the intensive care unit the child was intubated for airway and seizure control. Further diagnostic work-up by angiography revealed agenesis of the left coronary artery. After initial seizures, the boy's neurological recovery was complete. He subsequently underwent successful internal mammary artery in-situ bypass surgery to the trunk of the left coronary artery. One year after cardiac arrest, the patient had completely recovered with no physical or intellectual sequelae. A catheter examination proved excellent growth of the bypass and good cardiac function. Conclusions: This case illustrates the long term outcome after agenesis of the LCA while reiterating that prompt access to pediatric defibrillation may be lifesaving—albeit in a minority of pediatric OHCA. © 2017 Elsevier Inc. All rights reserved.
1. Background Non-traumatic, unspecific out-of-hospital cardiac arrest (OHCA) in children is a rare event, dreaded by paramedics and emergency physicians alike [1]. Reported incidence rates vary from 3 to 20 per 100,000 person-years. [8–11, 16–19]. Typically, collapse occurs just minutes after symptom onset [2] and even the most skilled teams may have little experience with prehospital Abbreviations: AED, Automated External Defibrillator; AHA, American Heart Association; ALCAPA, Anomalous left main coronary artery from the pulmonary artery; CPR, Cardio-Pulmonary Resuscitation; ECG, Electrocardiography; LCA, Left Coronary Artery; LMCA, Atresia of the Left Main Coronary Artery; OHCA, Out-of-Hospital Cardiac Arrest; OPALS, Ontario Pre-Hospital Advanced Life Support; SIDS, Sudden Infant Death Syndrome; VF, Ventricular Fibrillation; VT, Ventricular Tachycardia. ⁎ Corresponding author at: Center for Clinical Research, Unfallkrankenhaus Berlin, Warener Str. 7, 12683 Berlin, Germany. E-mail addresses: [email protected] (M. Weigeldt), [email protected] (S. Lahmann), [email protected] (K. Krieger), [email protected] (S. Buttenberg), [email protected] (V. Stephan), [email protected] (B. Stiller), [email protected] (D. Stengel).
resuscitation in this setting. According to estimates in Germany, emergency physicians encounter only one case of pediatric prehospital resuscitation in nine years [3]. Efforts were made to reach performance benchmarks of the American Heart Association (AHA) with regard to chest compression frequency and depth [4]. In cardiovascular-related OHCA, congenital abnormalities are the predominant cause in age groups 0 to 2 years (84%) and 3 to 13 years (21%) [5]. Coronary artery anomalies are the second most common cause of OHCA in the United States, and the second most common cause of sudden cardiac death in young athletes [2,6]. The prevalence of coronary artery anomalies is about 1% in the general population [6], and 2.2% in children with certified cardiovascular death [7]. Approximately half of all cases occur in patients without known heart disease [7]. Coronary artery anomalies are classified as to whether they cause obligatory, exceptional, or no ischemia. The most common congenital coronary artery anomaly is an aberrant outflow of the left main coronary artery from the pulmonary artery (ALCAPA). Because of low vascular resistance in the pulmonary artery, blood flows retrogradely from the right coronary artery via collaterals [8].
http://dx.doi.org/10.1016/j.ajem.2017.05.011 0735-6757/© 2017 Elsevier Inc. All rights reserved.
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011
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M. Weigeldt et al. / American Journal of Emergency Medicine xxx (2017) xxx–xxx
An anomalous origin of the left main coronary artery from the right sinus of Valsalva is commonly associated with sudden cardiac death [1, 8,9], caused by an obstruction of the coronary artery between the aorta and the pulmonary trunk in combination with a kinking or intrinsic narrowing at the anomalous ostium. Patients with this coronary variant tend to be asymptomatic until sudden cardiac death [8]. Atresia of the left main coronary artery (LMCA) is a rare condition but almost always associated with ischemia [6]. Again, blood flows retrogradely from the right coronary artery, and from smaller towards larger vessels [10,11,12]. This increases the risk of myocardial ischemia
and possible cardiac arrest in situations with enhanced metabolic need. Without symptoms like fatigue, angina, or exercise-induced syncope, however, patients are unlikely to undergo imaging and become diagnosed before any critical or life-threatening event [2]. 2. Case presentation This is a report on an apparently healthy 4-year old boy who, after 2 h of intensive play and exercise at an indoor playground, suddenly complained of stomach pain. Minutes after symptom onset he became
Fig. 1. ECG from AED with applied shock at 17:14:53 and subsequent restored sinus rhythm.
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011
M. Weigeldt et al. / American Journal of Emergency Medicine xxx (2017) xxx–xxx
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Fig. 1 (continued).
cyanotic and collapsed lifeless in his mother's arms. Employees at the playground made an emergency call and a police officer trained in basic life support immediately started cardiopulmonary resuscitation (CPR). Paramedics continued resuscitation in accordance with European Resuscitation Council guidelines. Automated external defibrillator (AED) (ZOLL AED Pro, ZOLL Medical Corporation, USA) rhythm analysis advised defibrillation. About 15 min after the collapse, a 50 J shock was applied, reconstituting sinus rhythm and circulation. An advanced life support team including an emergency physician arrived shortly
thereafter, providing mask ventilation and intravenous access. Pupillary response was positive and similar on both sides. The patient was transferred to a pediatric hospital, arriving there 40 min after the observed event. On admission to the pediatric intensive care unit, blood pressure was 120/70 mmHg, heart rate was 60–70 bpm, and peripheral capillary oxygen saturation was 92%, with a prolonged capillary refilling time. The patient scored 7 on the Glasgow Coma Scale, and demonstrated hyperreflexia with tonic-stretched extremities. Electrocardiography (ECG) showed a sinus rhythm with no signs of
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011
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Fig. 2. Coronary angiography before coronary bypass surgery showing agenesia of the left coronary artery.
Fig. 3. Coronary angiography after coronary bypass surgery with left internal mammary artery to the left anterior descending coronary artery.
ischemia or cardiac arrhythmia. Initial troponin-T was 294 pg/ml (reference limit b 14,0 pg/ml; SI: 0,294 μg/l) (Figs. 1, 2, 3). Medical therapy of a post-hypoxic status epilepticus failed, and intubation and mechanical ventilation was initiated. Presumed major brain ischemia as a result of cardiac arrest and possibly insufficient CPR was ruled out by a brain CT scan. Further MRIs revealed no signs of hypoxic brain damage although the electroencephalogram showed pathologic slow activity alterations with an intermitting right hemispheric focus. A pneumothorax after intubation was released by a chest-tube. Therapeutic hypothermia was terminated after 16 h; the core body temperature failed to decrease below 36 °C. Secondary analysis of initial AED-data revealed ventricular fibrillation. The arrest was attributed to cardiac origin. Holter ECGs showed no relevant rhythm anomalies, pauses, or signs of repolarization disturbance (i.e., QT-prolongation). Echocardiography demonstrated mild mitral valve regurgitation and a hyperdense papillary muscle as indicator of ischemia but without identifying the coronary artery anomaly. Troponin-T concentrations dropped to 152, 60, and 15 pg/ml (SI: 0,152, 0,06 and 0,015 μg/l) 3, 5 and 7 days after the collapse. The patient was safely extubated after three days. In his early recovery phase, he presented delayed responses and ataxia but gradually improved over the next few days. Two weeks after resuscitation he was discharged home for Christmas. Six days later he was admitted to a University Heart Center for further examination and surgical reconstruction. Cardiac MRI suggested a
lacking ostium of the left coronary artery (LCA). Coronary angiography confirmed this finding, with the left coronary artery system filled via collaterals from the right coronary artery. The cardiac surgeon found the left main coronary artery was atrophied to a fibrous cord. Coronary bypass surgery transposing the left internal mammary artery to the left anterior descending coronary artery was successful. One year after cardiac arrest, the patient recovered without physical or mental deficits. 3. Discussion The main causes for resuscitation in children are trauma, sudden infant death syndrome (SIDS), drowning, and respiratory failure. While cardiac causes of OHCA are widely regarded to be less common, their published frequency ranges from 3.2% to 29.0% [1,8-11]. An autopsy series conducted as part of the Ontario Pre-hospital Advanced Life Support (OPALS) study enrolled 414 children (mean age 5.9 ± 6.4 years) between 1992 and 2002 who had died after OHCA [11]. Cardiac reasons for death were testified by the coroner in 44 cases (10.6%, 95% CI 7.8–14.0%). OHCA in children is witnessed and bystander CPR is performed in about one third of all cases [12-15]. A shockable initial rhythm was reported in 3.8 to 19.0% of OHCAs. The likelihood of pulseless ventricular tachycardia (VT) and ventricular fibrillation (VF) increases with age [1,8,10,11,16-18]. Different eligibility criteria may explain the variability in shockable rhythm rates. Studies including SIDS show a lower
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011
M. Weigeldt et al. / American Journal of Emergency Medicine xxx (2017) xxx–xxx
frequency of shockable initial rhythms [17,18]. Witnessed arrest is associated with a higher prevalence of shockable rhythms, the highest found in children 9 to 17 years of age [28], probably because of delayed deterioration from VT/VF to asystole. [29] Like in adults, the most important prognostic factor determining the chance of survival is time to defibrillation [1,17]. Unfortunately, children under 8 years of age have a lower odds of being resuscitated with AED assistance [28]. General outcome after OHCA is poor, with b10% of all patients surviving the event. In addition, most children remain in a poor or vegetative neurological state after resuscitation [16-18]. Children younger than one year of age have the worst outcomes [19]. There is evidence that witnessed collapse significantly improves the probability of survival. However, data are inconsistent as to the role of bystander CPR [10,16,19]. Some studies suggest that a cardiac origin is associated with better outcomes [10,18]. An initial shockable rhythm (VF/VT) in children is uncommon but associated with a significantly higher rate of survival than asystole (30% versus 5%) as well as better neurological outcomes at hospital discharge [16-20]. In the illustrated case, all predictors of more favorable outcomes after OHCA were met. The child was older than one year, his initial rhythm was shockable, his collapse was witnessed and bystander CPR was performed immediately. Fifteen minutes after the emergency call, defibrillation started to breach ventricular fibrillation. In adults, longterm survival rates are N 70% when defibrillation is performed in VF/ VT within 3 min after collapse. Without AED, mortality increases by 7 to 10% per extra minute [1]. Although cardiac reasons are rare in pediatric OHCA, implementation of an AED should not be underestimated and cardiac origins should be kept in mind. Ventricular arrhythmias including ventricular fibrillation in children may occur because of congenital heart diseases, inheritable channelopathies, long QT syndrome or cardiomyopathies, myocarditis and cardiac tumors, as well as in structurally normal hearts and can lead to sudden cardiac death. Approximately 20 to 25% of sudden cardiac deaths are associated with exercise and sports [1]. Here is a chance for immediate resuscitation attempts, and the availability of public AED is potential lifesaving [29,30]. Three major review articles had identified case reports of LMCA published between 1955 and 2015 [13,15,21]. After excluding overlapping citations, these reviews contained 67 individual case studies, 35 of which were published between 2000 and 2014. Median age of patients was 38 (interquartile range, 8–61) years, most of whom were male (n = 41). Angina was reported the most prevalent symptom (30/67, 45%, 95% confidence interval [CI] 33–57%), followed by heart failure (15/67, 22%, 95% CI 13–34%). Coronary surgery was performed on 38 (57%) patients, and 12 subjects expired (18%, 95% CI 10–29%). Transthoracic echocardiography was occasionally proposed as a screening tool for congenital coronary artery anomalies, however, this is not substantiated by any data [22-24]. Like in any other coronary pathology, anomalies may be detected by angiography, computed tomography (CT) or magnetic resonance imaging (MRI) [12,15,23]. Typical treatment options encompass both coronary bypass surgery [15,21] and patch angioplasty [14,25-27].
4. Conclusions In this case of a four-year old boy with up to then unknown agenesis of the left coronary ostium, ventricular tachycardia (VT) caused by stress-induced cardiac ischemia was terminated by early AED. The causative pathology is uncommon and rarely leads to VT. Yet, international general resuscitation guideline recommendations proved effective and prompted adequate and successful emergency interventions. The rescue chain (bystander CPR, basic life support, AED, advanced life support) worked efficiently, allowing for subsequent reconstructive coronary surgery and full physical and mental recovery.
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Consent for publication Written informed consent was obtained from the patient's parents (legal guardians) for publication of this case report and accompanying images. Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Acknowledgements We thank the patient's family for their kind permission to allow us to publish our findings on their son. We thank Adrian Neuhold for editing the graphics. References [1] Maconochie IK, Bingham R, Eich C, Lopez-Herce J, Rodriguez-Nunez A, Rajka T, et al. European resuscitation council guidelines for resuscitation 2015: section 6. Paediatric life support. Resuscitation 2015;95:223–48. [2] Gajewski KK, Saul JP. Sudden cardiac death in children and adolescents (excluding sudden infant death syndrome). Ann Pediatr Cardiol 2010;3(2):107–12. [3] Eich C, Landsleitner B. Resuscitation - cardiopulmonary resuscitation in infants and children (paediatric life support). Anasthesiol Intensivmed Notfallmed Schmerzther 2016;51(3):196–207. [4] Sutton RM, Case E, Brown SP, Atkins DL, Nadkarni VM, Kaltman J, et al. A quantitative analysis of out-of-hospital pediatric and adolescent resuscitation quality–a report from the ROC epistry-cardiac arrest. Resuscitation 2015;93:150–7. [5] Meyer L, Stubbs B, Fahrenbruch C, Maeda C, Harmon K, Eisenberg M, et al. Incidence, causes, and survival trends from cardiovascular-related sudden cardiac arrest in children and young adults 0 to 35 years of age: a 30-year review. Circulation 2012;126(11):1363–72. [6] Villa AD, Sammut E, Nair A, Rajani R, Bonamini R, Chiribiri A. Coronary artery anomalies overview: the normal and the abnormal. World J Radiol 2016;8(6):537–55. [7] Vetter VL, Covington TM, Dugan NP, Haley DM, Dykstra H, Overpeck M, et al. Cardiovascular deaths in children: general overview from the National Center for the Review and Prevention of Child Deaths. Am Heart J 2015;169(3):426–37 (e23). [8] Bardai A, Berdowski J, van der Werf C, Blom MT, Ceelen M, van Langen IM, et al. Incidence, causes, and outcomes of out-of-hospital cardiac arrest in children. A comprehensive, prospective, population-based study in the Netherlands. J Am Coll Cardiol 2011;57(18):1822–8. [9] Gerein RB, Osmond MH, Stiell IG, Nesbitt LP, Burns S, Group OS. What are the etiology and epidemiology of out-of-hospital pediatric cardiopulmonary arrest in Ontario, Canada? Acad Emerg Med 2006;13(6):653–8. [10] Kitamura T, Iwami T, Kawamura T, Nagao K, Tanaka H, Nadkarni VM, et al. Conventional and chest-compression-only cardiopulmonary resuscitation by bystanders for children who have out-of-hospital cardiac arrests: a prospective, nationwide, population-based cohort study. Lancet 2010;375(9723):1347–54. [11] Ong ME, Osmond MH, Gerein R, Nesbitt L, Tran ML, Stiell I, et al. Comparing pre-hospital clinical diagnosis of pediatric out-of-hospital cardiac arrest with etiology by coroner's diagnosis. Resuscitation 2007;72(1):26–34. [12] Levisman J, Budoff M, Karlsberg R. Congenital atresia of the left main coronary artery: cardiac CT. Catheter Cardiovasc Interv 2009;74(3):465–7. [13] Musiani A, Cernigliaro C, Sansa M, Maselli D, De Gasperis C. Left main coronary artery atresia: literature review and therapeutical considerations. Eur J Cardiothorac Surg 1997;11(3):505–14. [14] Serraf A, Baron O, Nottin R, Lacour-Gayet F, Bruniaux J, Sousa Uva M, et al. Atresia or congenital stenosis of the left coronary ostium. Myocardial revascularization in 5 children. Arch Mal Coeur Vaiss 1993;86(5):587–91. [15] Tanawuttiwat T, O'Neill BP, Schob AH, Alfonso CE. Left main coronary atresia. J Card Surg 2013;28(1):37–46. [16] Atkins DL, Everson-Stewart S, Sears GK, Daya M, Osmond MH, Warden CR, et al. Epidemiology and outcomes from out-of-hospital cardiac arrest in children: the resuscitation outcomes consortium epistry-cardiac arrest. Circulation 2009;119(11): 1484–91. [17] Topjian AA, Berg RA, Nadkarni VM. Pediatric cardiopulmonary resuscitation: advances in science, techniques, and outcomes. Pediatrics 2008;122(5):1086–98. [18] Young KD, Seidel JS. Pediatric cardiopulmonary resuscitation: a collective review. Ann Emerg Med 1999;33(2):195–205. [19] Rajan S, Wissenberg M, Folke F, Hansen CM, Lippert FK, Weeke P, et al. Out-of-hospital cardiac arrests in children and adolescents: incidences, outcomes, and household socioeconomic status. Resuscitation 2015;88:12–9. [20] Nishiuchi T, Hayashino Y, Iwami T, Kitamura T, Nishiyama C, Kajino K, et al. Epidemiological characteristics of sudden cardiac arrest in schools. Resuscitation 2014; 85(8):1001–6. [21] Unzue L, Garcia E, Parra FJ, Palomo J, Friera LF, Solis J. Congenital atresia of the left main coronary artery in an adult: a rare anomaly with an unfavorable prognosis. Review of the literature. Cardiovasc Revasc Med 2015;16(8):498–502.
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011
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[22] Frommelt PC. Congenital coronary artery abnormalities predisposing to sudden cardiac death. Pacing Clin Electrophysiol 2009;32(Suppl. 2):S63–6. [23] Herlong JR. Congenital coronary artery anomalies. In: Garson A, Bricker JT, Fisher DJ, Neish SR, editors. The science and practice of pediatric cardiology. Baltimore: Williams & Wilkins; 1998. [24] Weigand JD, Gowda S, Lorber R, Madan N. Echocardiographic diagnosis of left main coronary artery atresia. World J Pediatr Congenit Heart Surg 2017;8(1):101–2. [25] Graidis C, Dimitriadis D, Psifos V. Congenital atresia of the left main coronary artery in an adult: an extremely rare anomaly. J Invasive Cardiol 2012;24(12):E325–7. [26] Ohba M, Matsuo K, Kabasawa M, Kito H, Asano S, Hirano M, et al. Surgical angioplasty for ostial atresia of left main coronary artery in child. Gen Thorac Cardiovasc Surg 2015;63(4):239–41.
[27] Varghese P, Leanage RU, Peek GJ. Congenital atresia of the ostium of left main coronary artery: a rare coronary anomaly, diagnostic difficulty and successful surgical revascularization. Congenit Heart Dis 2007;2(5):347–50. [28] Johnson MA, Grahan BJH, Haukoos JS, et al. Demographics, bystander CPR, and AED use in out-of-hospital pediatric arrests. Resuscitation 2014;85(7):920–6. [29] Young KD, Gausche-Hill M, McClung CD, et al. Pediatrics 2004;114:157–64. [30] Priori SG, Blomstrom-Lundqvist C, Mazzanti A, et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of suddencardiac death: the task force for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J 2015;36:2793–867.
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report Moritz Weigeldt, MD a,⁎, Sabine Lahmann, MD b, Konstantin Krieger, MD c, Sebastian Buttenberg, MD d, Volker Stephan, MD, PhD e, Brigitte Stiller, MD, PhD f, Dirk Stengel, MD, PhD MSc g a
Center for Clinical Research and Department of Anesthesiology, Critical Care and Pain Medicine, Unfallkrankenhaus Berlin, Warener Str. 7, 12683 Berlin, Germany Department of Anesthesiology, Critical Care and Pain Medicine, Unfallkrankenhaus Berlin, Warener Str. 7, 12683 Berlin, Germany Department of Internal Medicine and Cardiology, Unfallkrankenhaus Berlin, Warener Str. 7, 12683 Berlin, Germany d Department of Pediatrics, Sana Klinikum Lichtenberg, Fanningerstraße 32, 10365 Berlin, Germany e Department of Pediatrics, Sana Klinikum Lichtenberg, Fanningerstraße 32, 10365 Berlin, Germany f Department of Congenital Heart Defects and Pediatric Cardiology, Heart Center University of Freiburg, Mathildenstr. 1, 79106 Freiburg, Germany g Center for Clinical Research, Unfallkrankenhaus Berlin, Warener Str. 7, 12683 Berlin, Germany b c
a r t i c l e
i n f o
Article history: Received 8 March 2017 Received in revised form 9 May 2017 Accepted 10 May 2017 Available online xxxx Keywords: Pediatric Cardio-pulmonary resuscitation Out of hospital cardiac arrest - shockable rhythm Automated external defibrillator Coronary artery agenesis
a b s t r a c t Background: To illustrate a rare cause of out-of-hospital cardiac arrest in children, its differential diagnoses, emergency and subsequent treatment at various steps in the rescue chain, and potential outcomes. Case presentation: A 4-year-old boy with unknown agenesis of the left coronary ostium sustained out-of-hospital cardiac arrest. Bystander cardio-pulmonary resuscitation was initiated and defibrillation was performed via an automated external defibrillator (AED) shortly after paramedics arrived at the scene, restoring sinus rhythm and spontaneous circulation. After admission to the intensive care unit the child was intubated for airway and seizure control. Further diagnostic work-up by angiography revealed agenesis of the left coronary artery. After initial seizures, the boy's neurological recovery was complete. He subsequently underwent successful internal mammary artery in-situ bypass surgery to the trunk of the left coronary artery. One year after cardiac arrest, the patient had completely recovered with no physical or intellectual sequelae. A catheter examination proved excellent growth of the bypass and good cardiac function. Conclusions: This case illustrates the long term outcome after agenesis of the LCA while reiterating that prompt access to pediatric defibrillation may be lifesaving—albeit in a minority of pediatric OHCA. © 2017 Elsevier Inc. All rights reserved.
1. Background Non-traumatic, unspecific out-of-hospital cardiac arrest (OHCA) in children is a rare event, dreaded by paramedics and emergency physicians alike [1]. Reported incidence rates vary from 3 to 20 per 100,000 person-years. [8–11, 16–19]. Typically, collapse occurs just minutes after symptom onset [2] and even the most skilled teams may have little experience with prehospital Abbreviations: AED, Automated External Defibrillator; AHA, American Heart Association; ALCAPA, Anomalous left main coronary artery from the pulmonary artery; CPR, Cardio-Pulmonary Resuscitation; ECG, Electrocardiography; LCA, Left Coronary Artery; LMCA, Atresia of the Left Main Coronary Artery; OHCA, Out-of-Hospital Cardiac Arrest; OPALS, Ontario Pre-Hospital Advanced Life Support; SIDS, Sudden Infant Death Syndrome; VF, Ventricular Fibrillation; VT, Ventricular Tachycardia. ⁎ Corresponding author at: Center for Clinical Research, Unfallkrankenhaus Berlin, Warener Str. 7, 12683 Berlin, Germany. E-mail addresses: [email protected] (M. Weigeldt), [email protected] (S. Lahmann), [email protected] (K. Krieger), [email protected] (S. Buttenberg), [email protected] (V. Stephan), [email protected] (B. Stiller), [email protected] (D. Stengel).
resuscitation in this setting. According to estimates in Germany, emergency physicians encounter only one case of pediatric prehospital resuscitation in nine years [3]. Efforts were made to reach performance benchmarks of the American Heart Association (AHA) with regard to chest compression frequency and depth [4]. In cardiovascular-related OHCA, congenital abnormalities are the predominant cause in age groups 0 to 2 years (84%) and 3 to 13 years (21%) [5]. Coronary artery anomalies are the second most common cause of OHCA in the United States, and the second most common cause of sudden cardiac death in young athletes [2,6]. The prevalence of coronary artery anomalies is about 1% in the general population [6], and 2.2% in children with certified cardiovascular death [7]. Approximately half of all cases occur in patients without known heart disease [7]. Coronary artery anomalies are classified as to whether they cause obligatory, exceptional, or no ischemia. The most common congenital coronary artery anomaly is an aberrant outflow of the left main coronary artery from the pulmonary artery (ALCAPA). Because of low vascular resistance in the pulmonary artery, blood flows retrogradely from the right coronary artery via collaterals [8].
http://dx.doi.org/10.1016/j.ajem.2017.05.011 0735-6757/© 2017 Elsevier Inc. All rights reserved.
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011
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M. Weigeldt et al. / American Journal of Emergency Medicine xxx (2017) xxx–xxx
An anomalous origin of the left main coronary artery from the right sinus of Valsalva is commonly associated with sudden cardiac death [1, 8,9], caused by an obstruction of the coronary artery between the aorta and the pulmonary trunk in combination with a kinking or intrinsic narrowing at the anomalous ostium. Patients with this coronary variant tend to be asymptomatic until sudden cardiac death [8]. Atresia of the left main coronary artery (LMCA) is a rare condition but almost always associated with ischemia [6]. Again, blood flows retrogradely from the right coronary artery, and from smaller towards larger vessels [10,11,12]. This increases the risk of myocardial ischemia
and possible cardiac arrest in situations with enhanced metabolic need. Without symptoms like fatigue, angina, or exercise-induced syncope, however, patients are unlikely to undergo imaging and become diagnosed before any critical or life-threatening event [2]. 2. Case presentation This is a report on an apparently healthy 4-year old boy who, after 2 h of intensive play and exercise at an indoor playground, suddenly complained of stomach pain. Minutes after symptom onset he became
Fig. 1. ECG from AED with applied shock at 17:14:53 and subsequent restored sinus rhythm.
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011
M. Weigeldt et al. / American Journal of Emergency Medicine xxx (2017) xxx–xxx
3
Fig. 1 (continued).
cyanotic and collapsed lifeless in his mother's arms. Employees at the playground made an emergency call and a police officer trained in basic life support immediately started cardiopulmonary resuscitation (CPR). Paramedics continued resuscitation in accordance with European Resuscitation Council guidelines. Automated external defibrillator (AED) (ZOLL AED Pro, ZOLL Medical Corporation, USA) rhythm analysis advised defibrillation. About 15 min after the collapse, a 50 J shock was applied, reconstituting sinus rhythm and circulation. An advanced life support team including an emergency physician arrived shortly
thereafter, providing mask ventilation and intravenous access. Pupillary response was positive and similar on both sides. The patient was transferred to a pediatric hospital, arriving there 40 min after the observed event. On admission to the pediatric intensive care unit, blood pressure was 120/70 mmHg, heart rate was 60–70 bpm, and peripheral capillary oxygen saturation was 92%, with a prolonged capillary refilling time. The patient scored 7 on the Glasgow Coma Scale, and demonstrated hyperreflexia with tonic-stretched extremities. Electrocardiography (ECG) showed a sinus rhythm with no signs of
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011
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Fig. 2. Coronary angiography before coronary bypass surgery showing agenesia of the left coronary artery.
Fig. 3. Coronary angiography after coronary bypass surgery with left internal mammary artery to the left anterior descending coronary artery.
ischemia or cardiac arrhythmia. Initial troponin-T was 294 pg/ml (reference limit b 14,0 pg/ml; SI: 0,294 μg/l) (Figs. 1, 2, 3). Medical therapy of a post-hypoxic status epilepticus failed, and intubation and mechanical ventilation was initiated. Presumed major brain ischemia as a result of cardiac arrest and possibly insufficient CPR was ruled out by a brain CT scan. Further MRIs revealed no signs of hypoxic brain damage although the electroencephalogram showed pathologic slow activity alterations with an intermitting right hemispheric focus. A pneumothorax after intubation was released by a chest-tube. Therapeutic hypothermia was terminated after 16 h; the core body temperature failed to decrease below 36 °C. Secondary analysis of initial AED-data revealed ventricular fibrillation. The arrest was attributed to cardiac origin. Holter ECGs showed no relevant rhythm anomalies, pauses, or signs of repolarization disturbance (i.e., QT-prolongation). Echocardiography demonstrated mild mitral valve regurgitation and a hyperdense papillary muscle as indicator of ischemia but without identifying the coronary artery anomaly. Troponin-T concentrations dropped to 152, 60, and 15 pg/ml (SI: 0,152, 0,06 and 0,015 μg/l) 3, 5 and 7 days after the collapse. The patient was safely extubated after three days. In his early recovery phase, he presented delayed responses and ataxia but gradually improved over the next few days. Two weeks after resuscitation he was discharged home for Christmas. Six days later he was admitted to a University Heart Center for further examination and surgical reconstruction. Cardiac MRI suggested a
lacking ostium of the left coronary artery (LCA). Coronary angiography confirmed this finding, with the left coronary artery system filled via collaterals from the right coronary artery. The cardiac surgeon found the left main coronary artery was atrophied to a fibrous cord. Coronary bypass surgery transposing the left internal mammary artery to the left anterior descending coronary artery was successful. One year after cardiac arrest, the patient recovered without physical or mental deficits. 3. Discussion The main causes for resuscitation in children are trauma, sudden infant death syndrome (SIDS), drowning, and respiratory failure. While cardiac causes of OHCA are widely regarded to be less common, their published frequency ranges from 3.2% to 29.0% [1,8-11]. An autopsy series conducted as part of the Ontario Pre-hospital Advanced Life Support (OPALS) study enrolled 414 children (mean age 5.9 ± 6.4 years) between 1992 and 2002 who had died after OHCA [11]. Cardiac reasons for death were testified by the coroner in 44 cases (10.6%, 95% CI 7.8–14.0%). OHCA in children is witnessed and bystander CPR is performed in about one third of all cases [12-15]. A shockable initial rhythm was reported in 3.8 to 19.0% of OHCAs. The likelihood of pulseless ventricular tachycardia (VT) and ventricular fibrillation (VF) increases with age [1,8,10,11,16-18]. Different eligibility criteria may explain the variability in shockable rhythm rates. Studies including SIDS show a lower
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011
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frequency of shockable initial rhythms [17,18]. Witnessed arrest is associated with a higher prevalence of shockable rhythms, the highest found in children 9 to 17 years of age [28], probably because of delayed deterioration from VT/VF to asystole. [29] Like in adults, the most important prognostic factor determining the chance of survival is time to defibrillation [1,17]. Unfortunately, children under 8 years of age have a lower odds of being resuscitated with AED assistance [28]. General outcome after OHCA is poor, with b10% of all patients surviving the event. In addition, most children remain in a poor or vegetative neurological state after resuscitation [16-18]. Children younger than one year of age have the worst outcomes [19]. There is evidence that witnessed collapse significantly improves the probability of survival. However, data are inconsistent as to the role of bystander CPR [10,16,19]. Some studies suggest that a cardiac origin is associated with better outcomes [10,18]. An initial shockable rhythm (VF/VT) in children is uncommon but associated with a significantly higher rate of survival than asystole (30% versus 5%) as well as better neurological outcomes at hospital discharge [16-20]. In the illustrated case, all predictors of more favorable outcomes after OHCA were met. The child was older than one year, his initial rhythm was shockable, his collapse was witnessed and bystander CPR was performed immediately. Fifteen minutes after the emergency call, defibrillation started to breach ventricular fibrillation. In adults, longterm survival rates are N 70% when defibrillation is performed in VF/ VT within 3 min after collapse. Without AED, mortality increases by 7 to 10% per extra minute [1]. Although cardiac reasons are rare in pediatric OHCA, implementation of an AED should not be underestimated and cardiac origins should be kept in mind. Ventricular arrhythmias including ventricular fibrillation in children may occur because of congenital heart diseases, inheritable channelopathies, long QT syndrome or cardiomyopathies, myocarditis and cardiac tumors, as well as in structurally normal hearts and can lead to sudden cardiac death. Approximately 20 to 25% of sudden cardiac deaths are associated with exercise and sports [1]. Here is a chance for immediate resuscitation attempts, and the availability of public AED is potential lifesaving [29,30]. Three major review articles had identified case reports of LMCA published between 1955 and 2015 [13,15,21]. After excluding overlapping citations, these reviews contained 67 individual case studies, 35 of which were published between 2000 and 2014. Median age of patients was 38 (interquartile range, 8–61) years, most of whom were male (n = 41). Angina was reported the most prevalent symptom (30/67, 45%, 95% confidence interval [CI] 33–57%), followed by heart failure (15/67, 22%, 95% CI 13–34%). Coronary surgery was performed on 38 (57%) patients, and 12 subjects expired (18%, 95% CI 10–29%). Transthoracic echocardiography was occasionally proposed as a screening tool for congenital coronary artery anomalies, however, this is not substantiated by any data [22-24]. Like in any other coronary pathology, anomalies may be detected by angiography, computed tomography (CT) or magnetic resonance imaging (MRI) [12,15,23]. Typical treatment options encompass both coronary bypass surgery [15,21] and patch angioplasty [14,25-27].
4. Conclusions In this case of a four-year old boy with up to then unknown agenesis of the left coronary ostium, ventricular tachycardia (VT) caused by stress-induced cardiac ischemia was terminated by early AED. The causative pathology is uncommon and rarely leads to VT. Yet, international general resuscitation guideline recommendations proved effective and prompted adequate and successful emergency interventions. The rescue chain (bystander CPR, basic life support, AED, advanced life support) worked efficiently, allowing for subsequent reconstructive coronary surgery and full physical and mental recovery.
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Consent for publication Written informed consent was obtained from the patient's parents (legal guardians) for publication of this case report and accompanying images. Funding This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. Acknowledgements We thank the patient's family for their kind permission to allow us to publish our findings on their son. We thank Adrian Neuhold for editing the graphics. References [1] Maconochie IK, Bingham R, Eich C, Lopez-Herce J, Rodriguez-Nunez A, Rajka T, et al. European resuscitation council guidelines for resuscitation 2015: section 6. Paediatric life support. Resuscitation 2015;95:223–48. [2] Gajewski KK, Saul JP. Sudden cardiac death in children and adolescents (excluding sudden infant death syndrome). Ann Pediatr Cardiol 2010;3(2):107–12. [3] Eich C, Landsleitner B. Resuscitation - cardiopulmonary resuscitation in infants and children (paediatric life support). Anasthesiol Intensivmed Notfallmed Schmerzther 2016;51(3):196–207. [4] Sutton RM, Case E, Brown SP, Atkins DL, Nadkarni VM, Kaltman J, et al. A quantitative analysis of out-of-hospital pediatric and adolescent resuscitation quality–a report from the ROC epistry-cardiac arrest. Resuscitation 2015;93:150–7. [5] Meyer L, Stubbs B, Fahrenbruch C, Maeda C, Harmon K, Eisenberg M, et al. Incidence, causes, and survival trends from cardiovascular-related sudden cardiac arrest in children and young adults 0 to 35 years of age: a 30-year review. Circulation 2012;126(11):1363–72. [6] Villa AD, Sammut E, Nair A, Rajani R, Bonamini R, Chiribiri A. Coronary artery anomalies overview: the normal and the abnormal. World J Radiol 2016;8(6):537–55. [7] Vetter VL, Covington TM, Dugan NP, Haley DM, Dykstra H, Overpeck M, et al. Cardiovascular deaths in children: general overview from the National Center for the Review and Prevention of Child Deaths. Am Heart J 2015;169(3):426–37 (e23). [8] Bardai A, Berdowski J, van der Werf C, Blom MT, Ceelen M, van Langen IM, et al. Incidence, causes, and outcomes of out-of-hospital cardiac arrest in children. A comprehensive, prospective, population-based study in the Netherlands. J Am Coll Cardiol 2011;57(18):1822–8. [9] Gerein RB, Osmond MH, Stiell IG, Nesbitt LP, Burns S, Group OS. What are the etiology and epidemiology of out-of-hospital pediatric cardiopulmonary arrest in Ontario, Canada? Acad Emerg Med 2006;13(6):653–8. [10] Kitamura T, Iwami T, Kawamura T, Nagao K, Tanaka H, Nadkarni VM, et al. Conventional and chest-compression-only cardiopulmonary resuscitation by bystanders for children who have out-of-hospital cardiac arrests: a prospective, nationwide, population-based cohort study. Lancet 2010;375(9723):1347–54. [11] Ong ME, Osmond MH, Gerein R, Nesbitt L, Tran ML, Stiell I, et al. Comparing pre-hospital clinical diagnosis of pediatric out-of-hospital cardiac arrest with etiology by coroner's diagnosis. Resuscitation 2007;72(1):26–34. [12] Levisman J, Budoff M, Karlsberg R. Congenital atresia of the left main coronary artery: cardiac CT. Catheter Cardiovasc Interv 2009;74(3):465–7. [13] Musiani A, Cernigliaro C, Sansa M, Maselli D, De Gasperis C. Left main coronary artery atresia: literature review and therapeutical considerations. Eur J Cardiothorac Surg 1997;11(3):505–14. [14] Serraf A, Baron O, Nottin R, Lacour-Gayet F, Bruniaux J, Sousa Uva M, et al. Atresia or congenital stenosis of the left coronary ostium. Myocardial revascularization in 5 children. Arch Mal Coeur Vaiss 1993;86(5):587–91. [15] Tanawuttiwat T, O'Neill BP, Schob AH, Alfonso CE. Left main coronary atresia. J Card Surg 2013;28(1):37–46. [16] Atkins DL, Everson-Stewart S, Sears GK, Daya M, Osmond MH, Warden CR, et al. Epidemiology and outcomes from out-of-hospital cardiac arrest in children: the resuscitation outcomes consortium epistry-cardiac arrest. Circulation 2009;119(11): 1484–91. [17] Topjian AA, Berg RA, Nadkarni VM. Pediatric cardiopulmonary resuscitation: advances in science, techniques, and outcomes. Pediatrics 2008;122(5):1086–98. [18] Young KD, Seidel JS. Pediatric cardiopulmonary resuscitation: a collective review. Ann Emerg Med 1999;33(2):195–205. [19] Rajan S, Wissenberg M, Folke F, Hansen CM, Lippert FK, Weeke P, et al. Out-of-hospital cardiac arrests in children and adolescents: incidences, outcomes, and household socioeconomic status. Resuscitation 2015;88:12–9. [20] Nishiuchi T, Hayashino Y, Iwami T, Kitamura T, Nishiyama C, Kajino K, et al. Epidemiological characteristics of sudden cardiac arrest in schools. Resuscitation 2014; 85(8):1001–6. [21] Unzue L, Garcia E, Parra FJ, Palomo J, Friera LF, Solis J. Congenital atresia of the left main coronary artery in an adult: a rare anomaly with an unfavorable prognosis. Review of the literature. Cardiovasc Revasc Med 2015;16(8):498–502.
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011
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M. Weigeldt et al. / American Journal of Emergency Medicine xxx (2017) xxx–xxx
[22] Frommelt PC. Congenital coronary artery abnormalities predisposing to sudden cardiac death. Pacing Clin Electrophysiol 2009;32(Suppl. 2):S63–6. [23] Herlong JR. Congenital coronary artery anomalies. In: Garson A, Bricker JT, Fisher DJ, Neish SR, editors. The science and practice of pediatric cardiology. Baltimore: Williams & Wilkins; 1998. [24] Weigand JD, Gowda S, Lorber R, Madan N. Echocardiographic diagnosis of left main coronary artery atresia. World J Pediatr Congenit Heart Surg 2017;8(1):101–2. [25] Graidis C, Dimitriadis D, Psifos V. Congenital atresia of the left main coronary artery in an adult: an extremely rare anomaly. J Invasive Cardiol 2012;24(12):E325–7. [26] Ohba M, Matsuo K, Kabasawa M, Kito H, Asano S, Hirano M, et al. Surgical angioplasty for ostial atresia of left main coronary artery in child. Gen Thorac Cardiovasc Surg 2015;63(4):239–41.
[27] Varghese P, Leanage RU, Peek GJ. Congenital atresia of the ostium of left main coronary artery: a rare coronary anomaly, diagnostic difficulty and successful surgical revascularization. Congenit Heart Dis 2007;2(5):347–50. [28] Johnson MA, Grahan BJH, Haukoos JS, et al. Demographics, bystander CPR, and AED use in out-of-hospital pediatric arrests. Resuscitation 2014;85(7):920–6. [29] Young KD, Gausche-Hill M, McClung CD, et al. Pediatrics 2004;114:157–64. [30] Priori SG, Blomstrom-Lundqvist C, Mazzanti A, et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of suddencardiac death: the task force for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J 2015;36:2793–867.
Please cite this article as: Weigeldt M, et al, Pediatric out-of-hospital cardiac arrest caused by left coronary-artery agenesis with primary shockable rhythm. A brief report, American Journal of Emergency Medicine (2017), http://dx.doi.org/10.1016/j.ajem.2017.05.011